title: Micronutrient status in phenylketonuria.
abstract: Patients with phenylketonuria (PKU) encompass an 'at risk' group for micronutrient imbalances. Optimal nutrient status is challenging particularly when a substantial proportion of nutrient intake is from non-natural sources. In PKU patients following dietary treatment, supplementation with micronutrients is a necessity and vitamins and minerals should either be added to supplement phenylalanine-free l-amino acids or given separately. In this literature review of papers published since 1990, the prevalence of vitamin and mineral deficiency is described, with reference to age of treatment commencement, type of treatment, dietary compliance, and dietary practices. Biological micronutrient inadequacies have been mainly reported for zinc, selenium, iron, vitamin B12 and folate. The aetiology of these results and possible clinical and biological implications are discussed. In PKU there is not a simple relationship between the dietary intake and nutritional status, and there are many independent and interrelated complex factors that should be considered other than quantitative nutritional intake.
authors.affiliation: Nutrition and Metabolism Unit, Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium.
authors.forename: M
authors.initials: M
authors.lastname: Robert
authors.forename: J C
authors.initials: JC
authors.lastname: Rocha
authors.forename: M
authors.initials: M
authors.lastname: van Rijn
authors.forename: K
authors.initials: K
authors.lastname: Ahring
authors.forename: A
authors.initials: A
authors.lastname: Bélanger-Quintana
authors.forename: A
authors.initials: A
authors.lastname: MacDonald
authors.forename: K
authors.initials: K
authors.lastname: Dokoupil
authors.forename: H
authors.initials: H
authors.lastname: Gokmen Ozel
authors.forename: A M
authors.initials: AM
authors.lastname: Lammardo
authors.forename: P
authors.initials: P
authors.lastname: Goyens
authors.forename: F
authors.initials: F
authors.lastname: Feillet
mesh_terms: D000293:Adolescent; D000328:Adult; D000375:Aging; D002648:Child; D002675:Child, Preschool; D019587:Dietary Supplements; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D018977:Micronutrients / Q000008:administration & dosage / Q000172:deficiency*; D008903:Minerals / Q000008:administration & dosage*; D009751:Nutritional Requirements; D009752:Nutritional Status; D010349:Patient Compliance; D010661:Phenylketonurias / Q000150:complications / Q000178:diet therapy / Q000503:physiopathology*; D026681:Vitamin B 6 Deficiency / Q000209:etiology*; D014815:Vitamins / Q000008:administration & dosage*; D055815:Young Adult
chemical_list: D018977:Micronutrients; D008903:Minerals; D014815:Vitamins
keywords: Iron; Micronutrients; PKU; Selenium; Vitamin B(12); Zinc